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Growth deficits in children with sickle cell disease
Célia Maria Silva1, Marcos Borato Viana
1Hemoglobinopathy Out-Patient Clinic, Hemominas Foundation, Belo Horizonte, Brazil.
Insights
Children with sickle cell anemia (HbSS) show significant growth deficits, particularly in weight, over one year. Rapid red blood cell turnover may contribute to this impaired growth in pediatric patients.
Area of Science:
- Pediatric Hematology
- Growth and Development
- Sickle Cell Disease
Background:
- Growth deficits are prevalent in children with sickle cell disease.
- The underlying pathophysiology of impaired growth remains poorly understood.
- Prospective studies are limited.
Purpose of the Study:
- To prospectively collect anthropometric measurements in children with sickle cell disease over one year.
- To correlate these measurements with hematologic data.
- To investigate growth patterns in pediatric sickle cell disease.
Main Methods:
- Included 100 children under 8 years old: 73 with sickle cell anemia (HbSS) and 27 with hemoglobinopathy SC (HbSC).
- Standardized Z scores for weight-for-age (waz), height-for-age (haz), and weight-for-height (whz) were calculated.
- Compared measurements to the National Center for Health Statistics (NCHS) reference population.
Main Results:
- Children with HbSS showed significant decreases in waz (p=0.01) and whz (p=0.02) after one year.
- No significant decrease in haz was observed (p=0.48).
- Lower waz scores correlated with lower hemoglobin and higher reticulocyte counts (p=0.03, p=0.08).
Conclusions:
- Growth deficits are evident in children with sickle cell anemia (HbSS) even over short observation periods.
- Accelerated red blood cell turnover is a potential contributing factor to impaired growth.
- Anthropometric measurements did not significantly deteriorate in children with HbSC.
Background:
Growth deficits are common in children with sickle cell disease. Few prospective studies are available and the pathophysiologic basis for the impaired growth is not clearly understood. Our objectives were to collect data on anthropomorphic measurements of children with sickle cell disease prospectively followed for 1 year and to correlate them with hematologic data.
Methods:
One hundred children <8 years of age (73 with homozygous SS sickle cell anemia [HbSS] and 27 with hemoglobinopathy SC [HbSC]) were included. Standardized Z scores of weight for age (waz), height for age (haz), and weight for height (whz) were compared to the National Center for Health Statistics (NCHS) reference population.
Results:
At study entry, the means (standard deviation [SD]) of waz, haz, and whz were -0.69 (1.06), -0.65 (1.11), and -0.32 (1.00), respectively. After 1 year of study, children with HbSS presented a significant decrease in waz (p = 0.01) and whz (p = 0.02); the decrease in haz was not statistically significant (p = 0.48). The effect was similar for children older or younger than 24 months of age. The decrease in waz and whz was significant for boys but not for girls. After 1 year of follow-up, lower mean waz scores were observed among patients with lower hemoglobin concentration and higher reticulocyte count (p = 0.03 and p = 0.08). Hemoglobin concentration was higher in girls. The anthropomorphic measurements did not deteriorate significantly in children with HbSC.
Conclusions:
Growth deficits may be demonstrable in children with HbSS, even during a short period of observation. Fast red blood cell turnover may be partially responsible for the observed effect.
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